Pathobiology of pulmonary hypertension. Extracellular matrix.

Changes in the extracellular matrix underlie the structural and functional abnormalities in the vessel wall that lead to progressive pulmonary vascular disease. Studies are reviewed aimed at addressing the cellular and molecular programs that regulate the production of the extracellular matrix describing new ways to arrest proliferation and migration of smooth muscle cells and to induce apoptosis. The latter can lead to the reversal of pathology at least in experimental animal models.